Therapeutic Challenges for Symptomatic Portal Cavernoma Cholangiopathy

Abstract

Although transjugular intrahepatic portosystemic shunts are most frequently used for the management of portal hypertension, the surgical approach is preferred for symptomatic portal cavernoma cholangiopathy. We present the case of a 25-year old female patient with a portal cavernoma secondary to catheterization of the umbilical vein at birth. She had had two episodes of esophageal variceal bleeding, successfully treated by endoscopic banding. and an episode of acute cholangitis secondary to portal cavernoma cholangiopathy. Endoscopic sphincterotomy and biliary stenting were performed, and were followed by repeated episodes of biliary stent occlusion. The last biliary drainage procedure triggered a massive hemobilia. Since endoscopic therapy was ineffective, a surgical mesocaval shunt with graft interposition and splenectomy was performed with favorable outcome. In selected cases, the mesocaval shunting plays an essential role in the treatment of portal cavernoma cholangiopathy even in the era of interventional radiology.

Abbreviations: CBD: common bile duct; CT: computed tomography; ERCP: endoscopic retrograde cholangiopancreatography; IVC: inferior vena cava; LHD: left hepatic duct; MRCP: magnetic resonance cholangiopancreatography; PC: portal cavernoma; PCC: portal cavernoma cholangiopathy; SMV: superior mesenteric vein.